Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0023473 (chronic myeloid leukemia)
 18,916 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 46-year-old man was diagnosed as having chronic myelogenous leukemia (CML) in chronic phase in Dec. 1985. In Dec. 1987, anemia and leukocytopenia progressed, and the percentage of blast cells increased in the bone marrow. The blast cells were lymphoblastoid and positive for TdT. It was treated as a lymphoid crisis with vincristine and prednisolone, and complete remission was achieved. However, the blasts (11%) were observed in the bone marrow in Mar. 1988, and the chromosomal analysis revealed 46, XY, t (2q-; 11q+), t (9q+; 22q-) in 13 out of 20 cells. In June, the percentage of the blasts increased again, but chromosomal analysis showed a different karyotype, 46, XY, t(2p-; 11p+), t(9q+; 22q-) which was observed in 9 out of 10 cells. Then, myeloblastoid cells increased rapidly in spite of the chemotherapy in Dec. 1988. The chromosomal analysis showed 46, XY, 2p-, 7q-, 9q+, 11p+, 22q- in all analyzed cells. The rearrangement of the bcr gene could be detected by the Southern blotting. The blasts were positive for CD7, CD11, CD13, CD33, CD36, CD41 and CD42, suggesting that the blasts had the surface phenotypes of both myeloid and megakaryocytoid-lineage. This is a case with the mixed blast crisis that changed from the lymphoid to the myelo-megakaryocytoid in nature, in which three clonal evolutions were observed during the clinical course.
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PMID:[Mixed blast crisis with the cytogenetic evidence of three clonal evolutions]. 236 40

The blastic cell phenotypes of 26 cases of CGL in blastic phase were estimated and the patients were treated with different schemes. The following methods of typisation of the blast cells were used: cytochemical stainings (POX, Sudan B, PAS, nonspecific esterase), estimation of TdT activity, and in 11 patients the testing with monoclonal antibodies of VI series. Using these methods 10 patients (38%) with lymphoid form of the blastic phase, 11 (43%) with the myeloid type and 5 patients (19%) with undifferentiated type were diagnosed. In the group of lymphoblastic type a longer survival time and complete remissions were observed. High TdT activity in blastic cells did correspond with favourable response to Vincristin and Prednison. The introduction of TdT assessment into the diagnosis of CGL allows the cells to be classified more precisely, thus helping in defining the prognosis and in the choice of treatment programme.
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PMID:Diagnostic and prognostic value of the terminal deoxynucleotidyl transferase (TdT) activity in treating the blastic phase (bp) of chronic granulocytic leukaemia (CGL). 241 15

We report a case of blast crisis in chronic myelogenous leukemia (CML) in which T lymphocytic and megakaryocytic lineages were both involved. A 55-year-old male was initially admitted to Ehime University Hospital because of generalized lymphadenopathy. The morphological features of peripheral blood and bone marrow were consistent with chronic phase of CML. Cytogenetic studies of bone marrow and lymph node cells both showed the Ph1 chromosome with additional abnormalities. The patient was diagnosed as being in the extramedullary blast crisis of CML involving lymph nodes. After six months, blasts increased in bone marrow and peripheral blood. The phenotypes of lymph node blasts were positive for CD2, CD7 and TdT, but negative for CDw41 (platelet glycoprotein IIb/IIIa). On the other hand, those of peripheral blood blasts were positive for CDw41, but negative for CD2, CD7 and TdT. Chromosome studies of lymph node cells and bone marrow cells revealed 46, XY, inv(7) (p15q34), t(9;22) (q34;q11) and 46, XY, t(1;3) (q23:q21), t(9;22) (q34;q11), respectively. The rearrangement of T cell receptor beta chain gene was detected in lymph node blasts, but not in peripheral blood blasts. The identity of the rearrangement patterns of the breakpoint cluster region on chromosome 22 was detected in these blasts. According to these data, it was suggested that blast crisis of CML occurred in two distinct lineages, T lymphocyte and megakaryocyte.
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PMID:A case of chronic myelogenous leukemia with T lymphoblastic and megakaryoblastic mixed crisis. 254 79

Dual rearrangement of immunoglobulin and T-cell antigen receptor (beta, delta) genes was demonstrated in a case of Philadelphia chromosome-positive chronic myeloid leukemia (CML) in blast crisis. The blast cells, showing L2 morphology and high activity of TdT, expressed pre-B cell (CD19+, Ia+) and myeloid (CD13+, CD34+) surface antigens but lacket T-cell antigens (CD2-, CD7-). Cytogenetic studies on bone marrow and peripheral blood revealed the Phl chromosome in all metaphases analyzed, majority of which also had the additional chromosome changes, +8, +10, +21. Furthermore, molecular analysis of the breakpoint cluster region (bcr) on chromosome 22 showed a rearrangement, confirming the CML origin of the blast cells.
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PMID:Dual rearrangement of immunoglobulin and T-cell receptor genes in blast crisis of CML. 254 92

We applied a new technique to the detection of intracellular TdT in 26 leukemic patients, including 16 non-T acute lymphoblastic leukemia (ALL), four T-ALL, one T-lymphoblastic lymphoma in leukemia phase, one undifferentiated leukemia, one de novo lymphoblastic phase of chronic myeloid leukemia, and three acute monocytic leukemias (AMOL). Mononuclear cell suspensions were incubated in saponin to permeabilize the cell membrane. The cells were then stained by indirect immunofluorescence (IF) using anti-human TdT monoclonal antibodies and were analyzed by flow cytometry (FCM). The TdT results were compared with those obtained by biochemical TdT assay (26 cases), immunoperoxidase determination (PAP) (12 cases), and fluorescence microscopy (seven cases). The results obtained by PAP and fluorescence microscopy were 100% concordant with those obtained by FCM and biochemical assay. TdT determination by FCM allows the analysis of large numbers of cells in a fast, objective, and reliable manner, as compared with biochemical assay, PAP, and fluorescence microscopy determinations.
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PMID:Detection of terminal deoxynucleotidyl transferase (TdT) by flow cytometry in leukemic disorders. 264 40

Analysis at the DNA and RNA level revealed a mature genetic marker profile in a case of T type blast crisis of chronic myelocytic leukemia. T cell receptor beta chain gene rearrangement as well as T cell receptor alpha mRNA transcription was demonstrated in blasts of the malignant clone. Corresponding findings were obtained from immunological phenotyping. Blasts were found to be CD7+, CD1+, CD3+, CD4+, CD8+, and TdT- and classified as common/mature thymocytes. The presence of the breakpoint cluster region gene on chromosome 22 excluded the possibility of a second neoplastic process.
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PMID:T cell receptor alpha mRNA transcription in T-lymphoblastic transformation of chronic myelocytic leukemia. 325 50

The method to fix single cells in suspension and its application for the detection of TdT-positive cells by flow cytometry are described. In comparison to formalin-methanol fixation, formalin-acetone fixation resulted more formation of aggregated cells which caused non-specific staining. In our assay system 80% cells in human thymus were TdT-positive. Levels of TdT in normal human peripheral blood was 0.5 +/- 0.6% (means +/- 1 S.D., n = 50). A total of 104 patients with leukemia/lymphoma was examined for TdT. TdT-positive cells were detected in ALL (87.5% cases), AML (57.1% cases), CML-BC (58.3% cases) and ML (17.6% cases). Mean channel of fluorescence intensity of the TdT-staining in AML was significantly reduced in comparison with that in ALL. When antigen density of TdT was very low, fluorescence microscopy gave false negative results and flow cytometry could detect this dim fluorescence.
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PMID:Enumeration of terminal deoxynucleotidyl transferase positive cells in leukemia/lymphoma by flow cytometry. 329 65

TdT (terminal deoxynucleotidyl transferase) can be detected by radio enzymatic assay, biochemical assay in cell extracts, serum or plasma, and intracellularly in the smear by indirect immunofluorescent methods. The IgG fraction of anti-TdT serum is conjugated with fluoresceinisothiocyanate and used directly on the cytospin smears of methanol fixed bone marrow/blood smears. The mice thymocytes and peripheral mononuclear cells of healthy donors were used as positive and negative controls, respectively, for TdT. 64% of our cases of ALL were found to be TdT+. The lymphoblasts of L1 morphology (FAB classification) were more frequently positive for TdT as compared to blasts with L2 morphology. 71% of our cALLa positive blasts in acute lymphoblastic leukemias were TdT+ve as compared to 58% of T-ALL blasts. 75% of PAS positive ALL cases were positive for TdT as well. Only 57% of the cases when acid phosphatase showed unipolar positivity (T type) were positive for TdT. 12% of cases with acute myeloid leukemia (6/47) were TdT+ve and 33% of CML in blastic crisis had TdT+ve blasts. Biochemical assay and IF assay for TdT were in good correlation in our study.
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PMID:Terminal deoxynucleotidyl transferase in acute leukemias by direct immunofluorescence. 330 37

Alpha-2 interferon, produced in Escherichia coli using recombinant DNA techniques, was administered to 17 children with refractory acute lymphoblastic leukemia (ALL) in relapse, two children with TdT-positive, Philadelphia chromosome-positive chronic myelocytic leukemia (CML) in blast crisis, and one child with B cell (SIg+) non-Hodgkin's lymphoma (NHL) in a second extramedullary relapse. An initial 2-week intravenous (IV) phase of interferon was followed by a 3-month subcutaneous (SC) maintenance phase if patients had an objective response or disease stabilization without significant bleeding or infectious complications. When interferon dosages were escalated from 3 to 100 X 10(6) U/m2 in the first phase of therapy, there was rapid progression of disease in the first four patients treated, prompting a modification of the treatment plan. The last 16 patients enrolled received fixed dosages of interferon (ie, 10, 20, 30, and 50 X 10(6) U/m2 administered to four subjects each). One child with T cell ALL had an 11-month complete remission; the patient with lymphoma had a dramatic but brief response; three others (one CML and two ALL) showed disease stabilization for 3 to 6 months with a definite oncolytic effect in two of the three patients. The remaining 15 patients had progressive disease within 2 months and were removed from the study. Acute toxicity included a flu-like syndrome in all patients, increased serum transaminase levels in five, seizures in three (two cases temporally related to fever and one to a thrombocytopenic subarachnoid hemorrhage), and prolonged activated partial thromboplastin times in seven. This phase I-II trial of recombinant alpha-2 interferon demonstrated definite activity without dose-limiting toxicity.
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PMID:Phase I-II study of recombinant alpha-2 interferon against advanced leukemia and lymphoma in children. 345 76

The object of this study was to develop a flow-cytometric procedure for measuring terminal transferase (TdT) in leukemic cells by indirect immunofluorescence. We demonstrated the presence of TdT in an average of approximately 80% of the cells from 12 patients with ALL, one with CML in lymphoid blast crisis, and one with T-lymphoblastic leukemia. These results compared favorably to a separate slide test for TdT using an immunofluorescent or immunoperoxidase method. In the 21 patients with nonlymphocytic leukemias and in six normal donors we studied, less than a mean of 3% of the cells were TdT+. In 11 of 12 patients with ALL, the TdT+ cells also carried the HLA-DR antigen and in 8 of 12 cases of ALL the TdT+ cells also expressed the CALLA antigen. We concluded that the flow-cytometric method facilitates the measurement of TdT and may significantly increase the ability to diagnose residual and recurrent ALL leukemias.
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PMID:Terminal transferase in leukemias by flow cytometry. 346 Jul 28


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